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Published on: September 12, 2019
Acquired Omenn-like syndrome, a novel posttransplant autoaggression syndrome reversed by rapamycin
Donald C Vinh1, Khalid Bin Dhuban, Helen Mason
1Division of Infectious Diseases, Department of Medicine, McGill University Health Centre, Montreal, QC, Canada.
Abstract:
Graft-versus-host disease is uncommon in autologous hematopoietic cell transplantation (HCT) and is typically brief and mild. We report unusual, protracted, and severe Omenn syndrome-like autoaggression following autologous HCT. We identified a profound FOXP3(+) regulatory T cell defect that coincided with hyperinflammatory T cell responses which were reversible with rapamycin in vitro.
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